#HearingLoss

Almost half of all hearing loss has an underlying genetic cause. Late-onset hearing loss, which occurs after the acquisition of speech, may appear in generation after generation, often progressing to a severe or even profound loss. Or it may skip generations, passing the faulty gene along to unsuspecting offspring.

Those affected, even if they were aware that they might eventually lose their hearing, are usually part of the hearing world and do not know sign language. The loss may be severe enough to be disabling, even with sophisticated hearing technology. Preventing this loss, even when anticipated, has not been possible.

On December 20th, researchers at the Broad Institute of MIT and Harvard and the Howard Hughes Medical Institute published a study in the prestigious journal “Nature” that holds promise for prevention of hereditary loss.

Why do we care about a study on mice? Mice, like all mammals including humans, cannot regrow damaged hair cells. If prevention works on mice, it may work on other mammals. Mice are also the test subject for studies on regeneration of hair cells, which would allow a reversal of hearing loss.

The mutant gene, whimsically called Beethoven by researchers, is found in the hair cells of the inner ear. Because the gene is dominant, it takes only one to cause damage. That also means it may exist alongside a healthy copy of the gene. One of the challenges for researchers was to find a way to target just the mutated gene without disrupting the normal copy.

The technique the researchers used, called CRISPR, is a gene editing technique that the journal “Science” cited as the 2015 Breakthrough of the Year. I won’t try to explain the technique (or even the acronym) but here’s a link to a reader-friendly article in the L.A. Times that laid out the technique – and its potential dangers.

What distinguished the new study, according to the scientists, is that this is the first time a genome-editing protein has been ferried directly into the relevant cells to halt progression of genetic hearing loss.

Direct delivery of the protein allows “exquisite DNA specificity,” according to the press release. The specificity is needed to selectively disrupt the pathogenic copy of the gene without disrupting the normal copy.

Since this type of genetic hearing loss generally manifests as late onset, it would allow researchers to test suspected carriers of the defective gene (the Tmc1 gene) and to treat carriers. As co-senior author Zheng-Yi Chen, associate professor at Massachusetts Eye and Ear said, the later onset allows “a precious time window for intervention.”

The subjects of the study were 100 Beethoven-model mice carrying one copy of the defective gene and one normal copy. Untreated, the mice began to show hearing loss at four weeks, and by eight weeks were profoundly deaf (as measured by auditory brain-stem response). The treated mice, in comparison, responded to sound at about 65 decibels, the level of normal human speech.

Peter Barr-Gillespie, a sensory biologist at Oregon Health and Science University who was not involved in the study, praised it (in the “New Scientist”) as a “pretty significant piece of work.” He noted, however, that the decibel level at which the treated mice could hear was relatively loud compared to the hearing threshold in wild mice, which is 30 to 40 decibels. “It’s nowhere near the threshold of the wild-type mice, [but] the 10-15 decibels could make a huge difference in humans,” he said. “That sort of loss of hearing is very noticeable in people and could make for substantial improvements in quality of life.”

As always with gene editing, one concern is possible undesirable changes in the DNA. Senior co-author David Liu said the researchers had not observed “any off-target editing in the animal.” In the specific cells treated, they found only one modification, in an area not known to play a role in hearing. One major concern, however, would be the potential to develop cancer. Stephen Tsang, a clinical geneticist at Columbia, praised the study as “good for basic research“ (in “Axios”) but, like others, noted that there are many more steps to prove it safe and effective for humans.

This study showed that gene editing prevented, rather than reversed, hearing loss in mice. But for those affected by genetic hearing loss, it’s a promising step. “A lot of additional work is needed before this strategy might inform the development of a therapy for humans,” co-senior author David Liu said, “but at this stage, we’re delighted and excited that the treatment preserved some hearing in the animal model.”

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Hearing loss is often referred to as an invisible disability, because there are no telltale markers — no wheelchair, no white cane. It’s invisible even compared to Deafness, with its vibrant silent language.

NYPD Officer Daniel Carione and his attorney, Colleen Meenan, who successfully sued for the right to wear hearing aids.

For a long time, people with hearing loss wanted to keep it invisible. They wanted hearing aids no one could see, they pretended they could hear when they couldn’t. Even today hearing aid companies advertise: “So small no one will ever know you’re wearing them.” Hearing loss is for old people, or damaged people, and our culture values youth and health.

But as more and more of us use hearing aids – both because we are getting older and because we live in a noisy society – we want accommodations. We want captions on our TV’s and in movie theaters, we want hearing assistive devices that work in lectures and live theater. (The hearing assistive device that works best in large venues is the induction loop, hands down.) We want to be able to hear – even if it means “hearing” through captions – in an emergency room or hospital, in a courthouse, at a town hall meeting, at a house of worship, at a lecture, on an airplane, at a political rally, on public transportation.

We want to be able to work, and we want the accommodations that make that possible.

Hearing loss is a disability that prevents us from participating in corporate, municipal, religious, cultural, and educational life — unless accommodations are provided. Accommodations insure equal access.

We are guaranteed accommodations under the Americans with Disabilities Act. But if we want to claim those accommodations, we need to acknowledge not only that we have hearing loss but that it is a disability. (That’s the name of the act, after all.)

This notion was reinforced by the speaker at our HLAA-NYC chapter meeting last week. Dan Carione, a New York City police offer with an illustrious 28-year career, was forced to retire in 2011 when the NYPD decided officers could not wear hearing aids. He fought that ruling and won. (You can read about his four-year fight in an article published by Hearing Loss Magazine, or in a New York Times Op-Ed.) But before he had any legal ground to stand on, he had to make an important admission to himself.

“The Americans with Disabilities Act is not this heroic shield that falls from the sky and protects each and every person who may or may not be disabled,” he said. “You have to be disabled. That was very difficult for me to accept.”

Dan Carione does not look disabled. He was – and is – a powerful physical and intellectual presence. To use the word disabled about himself defied the visible reality. “God bless Colleen [his attorney Colleen Meenan],” he said. “One of the first things she taught me was to use the word disabled. It’s counter-intuitive. Counter-intuitive, it hit me in the head like a dart because I didn’t want to use the word disabled. But if you’re not disabled, the ADA can’t protect you.”

So if we want access equal to the access that hearing people have, we have to be open about our hearing loss. We have to acknowledge that it is a disability. That does not mean it’s disabling – it’s only disabling if we are denied the accommodations that make us equal.

For more information on living with hearing loss, see my books on Amazon.com.

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Getting hearing access for those who are deaf or hard of hearing is a little more complicated.

One accommodation for the hard of hearing is an American Sign Language interpreter. But only a small minority of those who need it, use it. — Getty Images

Trying to get accommodations for those of us who are hard of hearing or deaf can be a long, tough slog.

Two years ago, the U.S. Equal Employment Opportunity Commission (EEOC) filed a lawsuit against shipping giant FedEx Ground, charging it with discriminating against its deaf and hard-of-hearing employees and job applicants for years.

The EEOC alleged that the company violated federal law by failing to provide needed accommodations, such as closed-caption training videos, scanners that vibrate instead of beep, flashing safety lights and American Sign Language (ASL) interpretation.

FedEx filed a motion to dismiss the suit, but this year a federal judge denied the motion. The case is still under litigation.

All of which brings me to the sticky issue of accommodations — namely, that no one type works for all. There is no wheelchair ramp equivalent when it comes to hearing loss.

Although the FedEx case involves the specific needs of a particular workplace, the problems of hearing access challenge all of us and make asking for hearing access — even for something as simple as a better-equipped lecture hall — complicated. Which access do you ask for?

One accommodation that is routinely offered is an ASL interpreter. The problem is that only a small minority of those with hearing loss (less than 5 percent) use ASL. It’s no more helpful to most than an interpreter speaking Hungarian would be.

Among the most widely used accommodations, found in theaters, houses of worship and public gathering places, are infrared or FM headsets. The person with hearing loss borrows a headset from the venue. Sound, which travels through the regular sound system and then wirelessly to the headset, is amplified. Sometimes these systems work well. More often they are helpful to those with milder losses but not for anyone else. They also work only as well as the microphone. It the microphone is badly positioned, the headsets won’t deliver clear sound.

The technology that gets people most excited is induction looping. (Here’s a short video about it.) This, too, works through the venue’s regular sound system, and the sound quality is often excellent. It consists of a wire run around the perimeter of a room that transmits a signal, again wirelessly, to the audience member’s own hearing aid or cochlear implant, set to the telecoil setting. If a hearing aid does not have a telecoil, or the user doesn’t have hearing aids, headsets similar to those used for FM devices can be worn. Sometimes hearing people use them, just to hear better.

For some, the best option is captioning. This can be open captioning on a shared screen, similar to the captions on your television or subtitles on a movie. Or it can be closed captioning, sent to your personal device (an iPhone or iPad) or one provided by a theater. Generally this kind of captioning, called CART, which I described in some detail a few months ago, is live.

Scripts can also be scanned or typed into a new device being tested by Globetitles. It sends prescreened captions to personal devices, including computer and television screens, tablets and smartphones. The captions appear as red type on a black background, so they don’t bother others. You can see a sample by clicking on the Globetitles link.

Unfortunately, no single system fits all needs. Some think captioning serves the largest number of people. Others like looping because you don’t have to do anything except change the program on your hearing aid. Live captioning could be adapted to the kind of Sony glasses used in Regal Cinemas, or something like Google Glass could put captions right before your eyes.

Most venues will probably continue to offer one form of listening assistance (or none). But if that assistance does not serve a person with a hearing disability when another type of assistance would, that person can bring a lawsuit under the ADA.

As Lise Hamlin, the Hearing Loss of America Association’s director of public policy and state development, emailed me in a discussion about accessibility: “You might be able to make a case for both a listening system and captioning under the ADA. The relevant phrase is ‘effective communication.’ The key is providing effective communication to each person who needs it. If even one person is denied effective communication, they can file a claim.”

Most of us don’t want to get involved in a lengthy lawsuit — we just want to understand the play or the sermon or the mayor’s announcement or the community meeting or the visiting candidate. Is that so much to ask?

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Last month the prestigious National Academies of Sciences, Engineering and Medicine issued a report with a series of recommendations that, if adopted, could revolutionize the way consumers receive and pay for hearing health care in the United States.

Unfortunately, this report was not widely covered in the mainstream press, and although I wrote about this at the time, I’m also addressing it here, for those who may have missed this important development.

About 30 million Americans have hearing loss, but 67 to 86 percent of those who could benefit from hearing aids do not use them. The report called hearing loss “a significant public-health concern.” It is of course also of significant personal concern for those with hearing loss.

The June 2 report by the Committee on Accessible and Affordable Hearing Health Care for Adults represented input from experts in hearing research, public health, geriatrics and audiology, as well as from industry and consumer groups.

Consumers hope the recommendations will ultimately result in relief from the high cost of hearing aids, little insurance coverage and the limited availability of hearing professionals.

The committee listed 12 recommendations, but here are the four I consider most important.

Make buying a hearing aid easier by removing the Food and Drug Administration’s regulation requiring a medical evaluation or a waiver before a hearing aid can be purchased.

It’s not difficult for consumers to waive the medical evaluation, but many people feel they are required to see a doctor before buying a hearing aid. This is a stumbling block for many. As the report pointed out, the only time a medical visit is essential is if you display “red flag” conditions, including sudden or rapidly progressing hearing loss within the previous 90 days, acute or chronic dizziness, pain or discomfort in the ear, or a visible ear deformity.

Most primary care doctors do not conduct even a cursory screening for hearing loss, even among the elderly. Failure to recognize hearing loss can be a threat to quality health care. A patient may not hear the doctor clearly, for instance.

Make lower-cost, over-the-counter hearing devices more widely available by implementing a new FDA category for them.

This refers to personal sound amplification products, or PSAPs, and other less costly over-the-counter hearing devices.

Improve the compatibility of hearing technologies with other communications systems.

This refers not only to compatibility of hearing aids with other hearing assistive devices and technologies, but also to developing “open platform” hearing aid programming. This would allow health care professionals — or, eventually, the device owner — to be able to program the settings on any device from any manufacturer, which is currently not the case.

In many ways, the report echoes the highly critical findings of a White House advisory group’s report last October. The group, as reported in the New York Times, said that industry concentration and overregulation meant that hearing aids had “not experienced the dramatic reductions in price and increases in features that have been routinely seen across consumer electronics.”

“When compared in complexity to today’s smartphones costing a few hundred dollars each,” the report said, “even premium-model hearing aids are simple devices but can cost several thousand dollars.”

The response to the White House report, as well as to these new recommendations, has varied widely among industry, consumer and health care groups — “hailed by some groups and denounced by others,” noted audiologist Barbara Weinstein, writing on the website Hearing Health & Technology Matters.

But she urged all parties to work toward common goals. “It is a win-win for all stakeholders to close the gap between the proportion of persons with untreated age-related hearing loss and the proportion of those who enjoy a measurably high quality of life as the result of hearing health care interventions purchased through audiologists.

“We are part of the problem and part of the solution. Let’s change that balance and remain essential,” she said.

Dealing with the stigma of both hearing loss and aging at work can keep some employees from asking for accommodations — Thomas Barwick/Getty Images

Older workers with hearing problems face a double whammy: They’re dealing with the stigma not only of hearing loss but also of age. If they ask for accommodations on the job for hearing loss, they fear attention will be drawn to their age as well.

The Americans with Disabilities Act protects the rights of workers to ask for accommodations at work to help them hear more easily. However, research out of Oregon State University has found that older workers are less likely to feel there’s support for them to ask for that kind of help, because of worries they’ll be perceived as old by coworkers and managers.

Other research has shown that people with disabilitiesrefrain from requesting accommodations if they think coworkers would find the request “normatively inappropriate” — meaning not in keeping with the office culture. For instance, an office environment with a focus on maximizing profits like that in The Big Short or The Social Network is perceived as being much less likely to understand and tolerate a disability than would a nonprofit that prides itself on a more inclusive culture.

Research by David C. Baldridge and Michele L. Swift of Oregon State University’s College of Business, published in the journal Human Resources Management, studied the effect of age on such requests. Workers’ fear of seeming old, they found, may trump their fear of seeming to have a disability. Their findings were based on an email survey of 242 workers ages 18 to 69. Most had moderate to severe hearing loss.

Age itself has a negative stereotype in many workplaces, including the perception of “lower productivity, resistance to change, reduced ability to learn, and greater cost,” the authors wrote. “These stereotypes are often associated with fewer promotions, less training, lower performance ratings, and lower retention.”

But add disability to age and the stereotypes multiply. The older the person with a disability, the more likely they are to fear that others will attribute the request not to the disability, but to their age.

“Simply put,” the authors wrote, “people with disabilities appear to face a straightforward yet troubling question, ‘If I ask for a needed accommodation, will I be better or worse off?'”

In their discussion, the researchers advised managers and human resources personnel to realize that while many older employees may be eligible for and would benefit from disability accommodation, “these employees might also be particularly reluctant to make requests,” especially if they work in for-profit organizations or if the organization appears not to have others with disabilities.

When the disability is hearing loss, managers should make sure telephones have adjustable volume (and, I would add, be telecoil compatible). Large meetings should routinely include open captioning, which can also help employees with normal hearing follow what’s being said.

In an email interview, Baldridge said managers should think about the inclusion of persons with disabilities as “a normal aspect of diversity management.”

As the workforce ages, disability increases. The Bureau of Labor Statistics reports that about 26 percent of those ages 65 to 74 are in the workforce, and the majority are full-time workers. It’s in a company’s interest to have employees working at full capacity. If people are reluctant to ask for accommodations for a disability, their output and effectiveness are likely to suffer.

Americans are working longer than ever, whether by choice or necessity. It’s created a problem the workplace has tried to ignore: hearing loss among the millions of boomers still on the job

It’s 2016, which means that the first of the baby boomers turn 70 this year. 2.5 million of them. Having survived to 70, their life expectancy is now 85. That’s just the start. Ten thousand baby boomers will turn sixty-five every single day between 2011 and 2029, according the Pew Research Center, with similarly long life expectancies.

Many of them plan to continue working and many others will be involved in volunteer work. As of today, more than half of boomers are still on the job – that’s about 45 million baby boomers, ranging in age from 51 to 70. The total figure for boomers, according to the Census Bureau is 76 million.

By 2022, the Bureau of Labor Statistics predicts, nearly one-third of those 65 to 74 will be in the work force.One half of those 65 and over have hearing loss. Two-thirds of those 75 and over have hearing loss.

How is the workplace going to cope with this? So far, by ignoring it.

Very few workplaces offer accommodations for those with hearing loss. For instance, company wide meetings might be held in rooms equipped with hearing loops or with both an ASL interpreter and CART captions. Few businesses provide those accommodations. Audiologist Juliette Sterkens, who is HLAA’s Hearing Loop Advocate, thinks the omission is not deliberate on the part of employers: in an email she wrote that she was “convinced that most are unaware that their employees’ struggle.” Echoing a familiar comment about the invisibility of hearing loss, she added, “they may realize something is not right but they cannot put their finger on it.

For now, it falls on you – the hearing-diminished boomer – to hold your own.

Here are some tips.

*Get hearing aids. Unfortunately, corporate America drops the ball on this one too. Most company insurance plans do not pay for hearing aids, nor does Medicare.

*If you can’t afford hearing aids, try a PSAP. If you’re not sure what a PSAP is, start with this New York Times article: “Just Don’t Call Them Hearing Aids.” At one-tenth the average price of a hearing aid, they are very effective for those with mild to moderate hearing loss. Only 20% of those who could benefit from hearing aids use them. That number holds firm well into old age. Hearing aids work. So do PSAP’s. For most employees, either of these devices can correct hearing to nearly normal.

*If you are still having trouble despite hearing aids, tell your supervisor and your colleagues. And then tell them again and again. Hearing loss is invisible, and they’ll quickly forget they need to look at you when they talk, refrain from yelling on the phone, not expect you to hear them from across the room.

*Make sure your hearing aids have telecoils. By law all landline telephones must be hearing-aid compatible, which means that the inner workings won’t cause your hearing aid to buzz. If you flip to telecoil mode when making or receiving a call the reception will be far clearer. And if you happen to work in one of those rare places where meeting rooms are looped, you’ll be able to hear as clearly from the back row as you would from the front.

*If necessary, ask for accommodations. The easiest and most effective of these is a captioned telephone. A captioned phone won’t work for a trader on the floor of the stock exchange – the captions are too slow – but it will help a majority of those with hearing loss to follow a business conversation.

*If you work at an information counter or a cash register, or anyplace where you regularly interact with people, ask for a portable hearing loop. These are usually thought of as accommodations for customers with hearing loss, but they are equally effective for an employee with a loss.

*Use personal assistive-listening devices. If you need to regularly hear one or more speakers – at a daily meeting for instance, look into FM systems or Phonak’s Roger system. The FM systems work best for one on one conversations, but the Roger can be used with several microphones around a table, allowing you to hear all the speakers.

*If you feel you are being discriminated against because of your hearing loss, keep a record of incidents. These can turn out to be invaluable in retaining your job, or in getting a decent settlement if you are told you’re no longer needed.

No company will tell you they’re firing you because you can’t hear, but there are plenty of pretexts – the job is being eliminated, you’re “not a team player,” etc. – and you may be able to expose them for just that if you have kept a written record.

No one wants to get into a lawsuit, and the big guys usually win. So optimize your workplace experience, and keep your job.